"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘High Intracranial Pressure’

Since February 9th, Em has had 75 episodes that are seizure-like. Sometimes she has several a day. Sometimes she goes a few days between episodes.

They look like a seizure but certainly not exactly. Now, it is possible that some of these episodes could be a type of seizure and the upcoming EEG monitoring should help us figure that out. But, I got a suggestion that tipped me off to what I think is a more likely possibility and I am very VERY grateful to Paulette for suggesting it because I suspect it would have been a very long time before the doctors would have figured it out.

Complex Migraine (or Migraine with Aura) is what I am now convinced this is. Apparently, the aura of a migraine can look very much like a seizure or even a stroke. I think we didn’t notice the headache that typically follows the episodes because, let’s be honest, she pretty much always has a headache. Even when she complained that her headache was horrible afterwards – well, not only does she always have a headache, but headaches often follow a seizure, so it seemed to make sense. But, after doing some research, it makes more sense that this is a Migraine with Aura. Furthermore, I am wondering if she is specifically having basilar-type migraines, which would fall under the umbrella of Complex Migraine.

Initially, I was convinced the episodes were triggered by cervical instability – the first episode was in the car while she was wearing her hard collar because her neck was so unstable. She complained of her vision being “weird”, then was unable to move or talk and was in and out of consciousness – I thought she had just fallen asleep. She was barely able to walk once we got home 30 minutes later, then the symptoms resolved completely and she was fine. (Well, not fine, but the point is the symptoms wholly resolved until the next episode.) By the 4th episode, my records show her describing feeling like her head was “sitting on a burning spike”. Another time, she described the pain like her head was “expanding like a balloon when she breathes”. Looking back, she says she almost always had a worse headache after these episodes.

The length of a migraine aura is 5 to 60 minutes, while seizures typically last 2 or 3 minutes. Her episodes tend to last between 5 and 30 minutes – the longest being far too long for a seizure but right in the range of a complex migraine. In the early episodes, she tended to go limp for several minutes. Eventually, the episodes started looking more like a tonic clonic seizure – but not quite as violent. Just a lot of twitching and jerking and twisting stiffly and then going limp, often repeatedly. She often has trouble speaking, swallowing, and breathing during the episodes. Sometimes her eyes are closed, sometimes her eyes are open and dart back and forth the whole time. She sometimes has a black horizontal line through her visual field that eventually fades. She often can’t move her legs for several minutes after. Often, she is aware during, but goes in and out of consciousness. She is dazed, confused, and dizzy for 5 to 15 minutes after.

The headache part is hard because she has such layers of headaches. She has been dealing with high pressure headaches, then low pressure after the LP, then bouncing back and forth. So it has been hard to sift through all of that. But, she has been having what we now believe are legit migraines and they mostly follow these episodes, although she has had a few without the aura. They don’t respond to Diamox, she is very sensitive to light and sound, she is nauseated and the headache eventually passes a few hours later. She sometimes has multiple episodes in a day that result in the headache never going away and just getting worse with each episode. The headache is bilateral and originates at the base of her skull and wraps around to her temples. (These points are what makes me suspect a basilar-type migraine. If I am right, then my initial suspicion of brain stem involvement wasn’t entirely wrong.)

I have been keeping detailed records of the episodes but it finally occurred to me to keep track of her headache pain before and after the episodes. Typically, her headache is a 2 or 3 before and a 6 or 7 immediately after, increasing to an 8 or 9 within a few minutes.

These episodes are now mostly triggered by flashing lights: police lights, strobe lights, sunlight flickering through trees while driving, etc. There is one stretch of road on our way to Speech Therapy that is all woods – if it is a sunny day, she is almost guaranteed an episode. Watching TV shows with flashing lights on NetFlix often ends up triggering an episode. One episode was started by police lights that went by our house. (I never, ever realized how many flashing lights are in this world until now. It is ridiculous!) Fluorescent lighting is problematic, although I don’t think she has ever had an episode specifically triggered by it. She can sometimes avoid an episode by avoiding flashing lights: when driving or watching TV, she can avert or close her eyes and not go there. Not fool proof, but she does have some control. Sleep deprivation also seems to trigger them and sometimes they just happen with no obvious trigger.

The concussion from December probably has a lot to do with these episodes, in my opinion. I think the EEG monitoring will help us figure out any actual seizure involvement, hopefully. I do know that it is possible that the episodes that are more seizure-like and not quite fitting into the Complex Migraine symptoms might not be triggered during the monitoring or that epileptic activity might be harder to catch, so we may not have a conclusion on all of this. The important thing is that we get expert eyes on her. Epilepsy experts and hopefully migraine experts who really know the complexity that can exist and don’t try to slap a psychogenic label on her.

And, yes, there is a bit of irony here. Early in this journey, we were sent to a Headache expert who idiotically insisted Em was having migraines. We *knew* she wasn’t. We suspected high ICP and just wanted to try Diamox. Eventually we found a doctor who did prescribe Diamox and it made all the difference. So, for all these years, I have battled constantly to keep the right focus on her headaches.

But the situation has changed. I believe that damn concussion has radically changed the landscape and now we are dealing with a whole slate of new issues. So, if this is migraines, so be it and let’s address it. For that matter, if it is seizures, so be it and let’s address it.

I am hopeful that if we get the right people looking at this, we can figure it out and get her some relief. THAT is the important issue.

Finally, I just want to say how much I appreciate everyone who reads, comments and shares. Not only is it crucial for all of us to know that we are not alone on our journeys, but we all have knowledge that can help someone else who is struggling. My hope has always been that sharing our experiences here would help someone else but we have been so blessed by hearing about other’s experiences as well. We would be lost without you, truly. 🙂

So much has happened in such a short time, I can hardly remember what I have posted about and what I put off for later. In addition to the lumbar puncture, the blood patch, the ongoing high pressure issues and the likely CSF leaks, Em has been dealing with a concussion and that injured rotator cuff and, of course, what we presume to be CRPS.

Shoulder Injury

PT dismissed her because she was struggling intensely with the most basic exercises she was given. At the time, we were suspecting that her neck had become unstable and that was a big concern – for some reason people get a little jumpy when you mention her neck is unstable! Fortunately, a lot of the issues that we assumed were down to instability eased up when they did the lumbar puncture, easing the high pressure.

So, the plan was, talk to the specialists at Cinci and wait and see. In the meantime, her neck is better and she has been able to gain a lot more use of her shoulder and is out of the sling. At this point, we will just carry on and hope the shoulder doesn’t get reinjured. In a perfect world, we would be able to focus our energy on her shoulder but, as we are far from a perfect world, there are too many other things to worry about. Sadly, since it seems to be improving, her shoulder is low on the list of priorities at the moment. I am just glad she has use and motion back and has less pain.

Concussion

She is doing better, but still not well. I think I mentioned that she fell and hit her head (again) on the 9th, which worsened the memory loss and balance problems. That was a little scary, to be honest but the doctor felt like the second injury should not have been enough to cause any damage and waiting it out was the thing to do. We have just made it a priority to protect her head as much as possible and keep her safe until she is steady again. Some of our precautions she doesn’t particularly appreciate, but protecting her brain is non-negotiable.

Her balance has improved from what it was after bumping her head that second time, but it is still not great. She is using her wheelchair when we are out in public. That is frustrating for her, but, I can tell you that I am so grateful we have that wheelchair in times like this.

She has started speech therapy to help rehab her brain. She had her second visit yesterday and will go again tomorrow. Her memory loss is better – she is able to recall details much better than before – but she is still incredibly frustrated when she is searching for a word and just can’t pull it up. And she is bemused at what her brain does recall and at how odd it feels to not know she knows something until she suddenly remembers.

Right now, we are still being guided by her symptoms – she can do the speech exercises in varying chunks of time before she starts hurting. Sometimes she can go straight through for a considerable length of time before her head starts hurting and other times she hits the wall sooner. This week she is going to write answers on a worksheet with various questions and we will see how that goes. She had a disconcerting time a couple days after the second bump when she realized that her brain was sort of disconnected to the actual process of writing. She said it felt like a reflex but that her brain wasn’t really doing it.

We go back to the concussion doctor on the 5th and she will have neuropsych testing done. I think she is definitely better and continuing to improve: we are all happy with the progress she is making, but this is all new ground for us and it will be good to have knowledgeable eyes on her.

She did sit in her room and play her guitar a couple days ago and that was a wonderful sound. It has been a while and she is just now getting to the point of being able to remember the cords and lyrics that once came so easily to her. Music is such a part of her life – being able to play again is a huge morale boost.

CRPS

Way back at the beginning of December, we saw the pain doctor and emphatically expressed our need for help. (Sort of a comin’ to Jesus type event.) Bless him, he spent an hour with us on a day we weren’t even scheduled to see him and his waiting room was packed.

Anyway, I had taken in a referral form for a doctor I wanted him to send us to – a neurologist specializing in neuromuscular diseases at OSU medical center. I had filled the form out as fully as I could (and told them what to put in the lines they needed to fill out) in the hopes that there would be no excuse to not promptly refer us. It did take a polite reminder but they did eventually get us referred. Honestly, I wasn’t even sure if this doctor would see Em since she is 17 so it was a shot in the dark. His office called to ask some questions and clarify the situation and to inform us that he normally wouldn’t see anyone under 18 but that he would consider it.

Thankfully, he is willing to see her and we see him on the 15th of March. I am cautiously optimistic that he will be helpful in giving her a diagnosis and figuring all of this out. Recently, her lab results got a little more complicated and it is doubly good that we are going to him – I will get to that issue in a just a minute. I hear he is very smart and very kind so I believe we are on the right track and I am beyond grateful he is willing to see her.

New Diagnoses

Em has had a ton of labs done – between the CSF testing from the lumbar puncture and the blood work that has been done – we have at least been able to look at a lot of things that can be ruled out or addressed. We saw the neurologist last week and I was not surprised to hear him say Em is beyond him and needs someone with more knowledge than he has. He has absolutely done right by her for 4 years, so that was a little emotional. He will be available if we need him, but we need to get somewhere with more knowledge.

He was concerned – not overly but said it needs to be looked at – with one of her labs. The one test shows some markers for MS. She has three bands and four indicate MS. Now, he doesn’t think she has MS and I don’t think she has MS. I just think her entire nervous system is on fire and there is nothing at all conclusive about that test in regards to an MS diagnosis. However, she needs to be evaluated by someone who can handle all of her complexities. Fortunately, we already have an appointment with a doctor specializing in neuromuscular disease. That is exactly where we need to be and I doubt we would be able to get a sooner appointment anywhere. So, it all seems a bit Providential when I look at it.

Also, the doctor ordered an additional blood test which has come back elevated, indicating Sarcadosis. Am I surprised that at this point she is showing signs of an autoimmune issue? Not at all. Truly, I am just surprised she hasn’t shown any autoimmune signs previously. Again, and almost as always, she has enough symptoms that sort of tick the boxes for one thing but that also overlap with about a dozen other things. So, who knows. She also had a lung x-ray and we are being referred to a pulmonologist. Again, I am not getting too worked up about this – if it is sarcadosis, it is treatable. If it isn’t, the symptoms (which, if they exist, are being masked by everything else that is going on) are certainly explained by everything else she is dealing with.

ER Complaints

We have taken our complaints about the whole lumbar puncture/ blood patch/ ER/ latex exposure debacle to administration and we are, fortunately, being taken seriously. So far, profuse apologies and some ideas on how to make sure this never happens again – not to us or anyone else – have been offered. I am not going into details right now, because this is, after all, my husband’s employer, and I want to tread lightly but suffice it to say they are doing right by us and I think we will be satisfied by the time it is all done. I am very hopeful that this will be an opportunity to share awareness about Emily’s rare conditions and help foster an atmosphere that better understands how to treat complex, chronic illness. Which, in the end, is what matters to us.

One Last Thing

Finally, I would like to ask for your prayers for my mom. She has been diagnosed with breast cancer and will be having a mastectomy in the near future. But, she has great doctors and her prognosis is good, so, even though this is a blow, we have faith that this is all going to be ok.

So those are the highlights from our crazy life right now. If it all sounds exhausting, it is! But, we are hanging in there and trying to keeping our sanity intact.

I really wish our lives were boring enough to be able to cover events in a single post, but, alas, that is not the case. Have been needing to split posts into multiple parts a lot lately and probably will for some time to come…

When I left off last, Em had had a lumbar puncture and, although I asked for it and the doctor ordered it, the concurrent blood patch was not done. I asked for the blood patch to prevent the very likely problems that were going to occur after the LP – a blood patch simply helps stop a CSF leak from the LP site.

Em did fine after the LP, in fact, her head was pressure free for the first time in a long time. But, unsurprisingly, she started having problems by that evening. Her back was extremely painful and she was enduring a spinal headache – low pressure. She also appeared to have developed another leak in that same ear where the first presumed leak was and that probably added to the low pressure issue. Sitting up was painful, so she laid flat for most of the weekend. We dutifully waited out that time (as ordered) to get through the window until she got to the point where she was either willing to go to the ER or until the doctors would take her seriously.

Monday morning (the 18th) Radiology called to check on her and were concerned with her symptoms and told us to call the neurologist. I did and played phone tag with them for two days. When I finally talked to them, they said to either go to the ER or call the pain doctor. I called the pain doctor and was told he was not in and they had no answers for us.

So, I informed a very reluctant and miserable Emily that we had to go to the ER. Have you ever felt too bad to go to the ER?? (I know many of you know how that is.) Well, that is where Emily was Tuesday afternoon.

When we got to the ER, they took her seriously but likely because we were able to say the neurologist sent us. Which is why I waited until we had their word to go: it can get ugly when we don’t jump through that hoop. Anyway, they took her seriously and quickly got her into a room and got an IV and pain meds started.

Here, it is probably worth mentioning the thought process of the other parental unit in this situation. You know I was angry and frustrated. My hubby was livid that they did not do the blood patch along with the LP and forced us to the ER resulting in yet another bill. So, he was already angry that we had to drag Emily out to the ER, in the bitter cold, on snowy roads, when it likely could have been prevented.

The ER doc walked into this minefield when she came in and said Em needed a blood patch but…

Well, it isn’t considered an emergency procedure so if there were no anesthesiologists available, we would have to just go home and schedule it for the next day. You can imagine how this went over with us.

Fortunately, the anesthesiologist on call was willing to come back in even though she had just gone home. I appreciated that – giving credit where credit is due.

When she got around to doing the procedure, she snapped on the gloves that were in the epidural kit that had been sent up. Unfortunately, those gloves were latex. You may remember that Emily is severely allergic to latex, to the point of if one glove was laying in the corner or if latex had been used in that room for the previous patient, she will have a reaction. Air born latex particles will cause a serious reaction very quickly. I mean, no, she hasn’t died yet from it, but she has taken Benedryl many times and there have been times it has been scary. I would really prefer we not have to prove how bad it is through her death.

So, Em is sitting on the edge of the bed, in pain, half naked, prepped for the procedure, scared out of her mind because she hates needles and vividly remembers how much the LP hurt and has been told this is going to be the same. I am not paying attention to anything but Emily at that point. I am in front of her holding her up, because I know she cannot hold herself up. She looks at me and asks if those gloves are latex. I look over her shoulder and ask, mostly to humor her, because they won’t make that kind of mistake, right?

When I ask about the gloves, the doctor stops in horror and says, oh crap. (Or something like that. My mind was racing at that point but that was the general idea.) And rips them off, apologizing. I look back at Emily and ask if she is ok. She says, “Not really.” So I tell the doctor she needs Benedryl. She assures me she didn’t touch her with the gloves and was horrified to find out that just taking them out of the package was enough to causes a reaction. So, she wraps everything up in the now unusable kit and rushes it out the door and gets the nurse, who brings in Benedryl. (Funny story, she brings it in with a syringe and Emily, in the midst of an allergic reaction, is like, hey, I have my own Benedryl in my bag, no need to give me a shot. I am good. Needless to say she was relieved to find it was going to go directly into her IV, although she was understandably suspicious until it was done.)

At this point, I am past livid. Especially when I realize they hadn’t put an allergy bracelet on her when they checked her in. Would it have made the difference? Honestly, I doubt it. But that isn’t the point, is it? And why does this ill child have to save her own life?

So, they have to order a new kit and leave her sitting, miserable, freezing, scared, and feeling like they had just tried to kill her, for far too long. Finally, they get the new kit and the doctor and the phlebotomist both come in. The phlebotomist sort of joked that the hospital is a latex free facility and they must have found the only pair of latex gloves in the place. I call BS on that because I know better. Their system failed at every step of the way and could have killed her. This is being addressed.

The procedure was, well, not fun. The doctor was trying to get her numb enough to do the procedure but never really succeeded. They took a huge amount of blood (20 mls) pretty quickly, too quickly for her. If I had not been holding her up, she would have been on the floor. You can’t take that amount of blood from a POTS patient without some effect. I thought she needed more fluids afterwards but she just wanted to go home so I didn’t push that issue, although I wish I had.

They gave her time to rest after the procedure and then basically kicked us out. I asked for them to do something for her pain, which was now as bad or worse than it was when we arrived, because we had a 45 minute ride home and she was in agony. They had given her morphine, a couple hours before and were not inclined to give her anything else. They offered her a pain pill, half of what she already takes. The doctor came in and explained since she didn’t know exactly what was going on, she didn’t want to throw narcotics at Emily, which she ironically did anyway. She also implied that we would be violating our contract with the pain doctor if we pushed the issue.

In the end, we had a child who was in more pain than she was to begin with, making a liar out of me. I promised her they would help, that they would treat her pain. Once again, they have bolstered the wall between this ill child and the care she needs by showing me to be a liar and refusing to take her seriously.

These experiences leave us battered and bruised, especially when they should have been prevented. There will be more to this story: we are not going to accept this kind of treatment without making some waves. The latex exposure is huge. Huge. That needs to be addressed. Because my husband works at this hospital, our insurance forces us to do everything possible there. If her specialists at Cincy order blood work or an MRI, can’t do it there, have to go to our local hospital. If she needs to go to the ER, we have to go there. But, they clearly are not providing a safe place for her to be treated. So, yes, this one is a hill worth dying on. They either need to get their act together or allow us to take her somewhere where she will be safe.

We also are going to fight the policy that prevented the blood patch being done along with the LP. I am not stupid – I get that the two procedures are done by different departments and it is complicated, but are you telling me it is impossible to schedule this? Seriously.

At best, this is a situation where the right hand doesn’t know what the left hand is doing. At worst, well, I hate to speculate about profit, but there it is. Then there is the fact that her doctor ordered the blood patch. Ordered it. I was not aware that a doctor’s order could be viewed as a suggestion. If they couldn’t do it, it was on them to contact him so he was aware. They did not call him, neither before or after, I have been told by him personally. Again, their system failed this child at every point.

So, we are beyond angry. There is absolutely no reason any of this needed to happen. To be told they can’t do the blood patch along with the LP and that we just have to go to the ER if there is a problem, only to find out the blood patch is not an emergency procedure, is mind boggling. To be told by the pain doctor’s office they have no answers for us. The whole thing is a nightmare, to be honest.

On the upside, I think the blood patch did help. Her pressure is normalizing (read: getting high again) and she is trying to manage the increasing pressure with her Diamox but not taking too much so it dips down into low pressure. That is a juggling act to be sure. The procedure did not help her back pain, but that is not too surprising. She is still in enormous pain – every movement is painful, but at least the blood patch wasn’t pointless.

We were able to get in to see the neurologist on Friday. That was a minor miracle. And there are other things going on as well, so I will be posting about all of that, trying to catch up. And, with any luck, there will be more to report on how our complaints are received. I don’t want anybody fired, although Emily and I have thought it would be useful to make everyone who had a part in the whole latex portion wear a scarlet “A”. I am thinking it would be an effective deterrent, but I suppose I won’t push that since HR might object.

Honestly, I just want to educate the people providing my daughter’s care. I want to help them be better. I want them to take her problems seriously. I want them to take chronic illness and chronic pain patients seriously. I want policy to change – how many people have suffered and had no recourse like we do? It is not ok. I want to help them offer the care my kid needs. But, there are things that need to change and we are going to do our best to see that happen. In the meantime, Emily is slowly recovering – her physical wounds will heal but her emotional ones will take longer, I think.

Or two or three or…. whatever. As bad as the last 6 months have been, this past week or so has been especially harrowing.

Em hit her head last weekend. Again. Not bad but bad enough to bring on more memory loss and very poor balance. And she had been having this weird thing where she felt like her throat was collapsing, which was more than a little freaky. Along with the latest head injury came worsening headaches and a very unstable neck. Just like 5 years ago, she was having all kinds of problems and I started assuming many of them were from cervical instability. And that was not a pleasant thought.

The concussion doc was willing to see her, but we agreed that there was nothing he was going to be able to do – time to heal and protecting her head from further injury is what was needed. So, we will see him at her follow up February 5th and wait and see. He is a phone call away.

We decided that some blood work – particularly to check her CO2 level but also a couple other things – would be a good idea. So I called the Neurologist to ask for that. I was not surprised when the answer I got back was that they were ordering blood work and a lumbar puncture.

For more than 4 years, we have avoided lumbar punctures. Not only was I aware that they might not be completely conclusive regarding high pressure but EDS folks are delicate enough that even this relatively easy procedure should not be done lightly. Once I found the Driscoll Theory, I was 100% comfortable doing a trial of Diamox instead of an LP. If Em responded to Diamox, then it would logically follow that her headache was the result of high pressure. We found one doc who was willing to try and the rest is history. The headache she had had for 2 years was gone in a few hours after that first dose. For almost 4 and a half years, we have managed her headache with Diamox, eventually having to increase her dose and taking it 3 times a day rather than once, and we have managed her CO2 level (which allows the Diamox to work) with baking soda tablets. We had tried to skate around the issue with new docs, endured some eye rolling when we admitted she was being treated for high pressure but had never been actually tested for it and just generally crossed our fingers hoping no one would push the issue. It was all fine for 4 years.

But now, clearly something had changed. My suspicion was it was the head injuries but she probably had a CSF leak (in her ear) in December before the concussion, so maybe the headache is coincidental or cumulative or something, but the bottom line is what we had been doing was no longer working.

In my mind, there were 3 possibilities: she needed more Diamox to control the high pressure, her CO2 level was low which would mean she needed more baking soda tablets to bring it up so the Diamox would work, or there was something else entirely going on, like cervical instability. At any rate, I knew in my heart the time for an LP had come. So, I agreed but asked for a couple things to hopefully make the procedure easier and more successful. I wanted them to use an atraumatic needle, to minimize damage and leaking. And I asked that they schedule a blood patch to be done immediately after the LP, instead of waiting until she had problems. Because, let’s be real, there was a nearly 100% chance she was going to have problems.

The doctor’s office ordered both without issue, so I was happy.

A nurse from Radiology called to pre-register her for the procedure and asked a ton of questions about EDS and her medical issues. She was awesome. She promised to check into the special needle – she had never heard of such a thing before – and did say she wasn’t sure about doing the blood patch because they normally don’t do them but that she would ask the doctor and if she could do it, she would because Em is clearly a special case. So I was content that I had done everything I could to make the LP as safe and easy as possible. When I heard nothing back, I assumed the blood patch was a go.

Em was not thrilled – anything involving a needle is not her idea of fun – but as we waited for Friday I started thinking it couldn’t come quickly enough. Her headache was a 12 (on a scale of 1-10) when her Diamox wore off and a 9.5 when it was working. Clearly there was a problem: she was miserable. We got the blood work that I asked for done and were able to slide in blood work they needed before the LP at the same time, so we minimized the needles for her. When even a simple blood draw is challenging, is it any wonder we were nervous about a lumbar puncture?

We get there on Friday morning and once she is in the room and has been given some Zanax to help relax her, I find out that they will not be doing the blood patch. LPs are done by Radiology and blood patches are done by Anesthesiology.

“Sorry, I don’t know why you were ever told it was possible. It is our policy to not do them here”,” the nurse said. “If she has problems, you will need to call your Pain Management doctor or go to the ER.”

Now, at this point, I am starting to fume. No one has called me to tell me this and I was given the impression it was happening. So, when she said that, I just gave her a look. The Look. Which she correctly interpreted. She sheepishly acknowledged that, since it would be over the weekend, we would, in fact, not be able to get a hold of the pain doc and that we would just need to head to the ER. I told her I understood this was not her fault, but that we had specifically asked for the blood patch to avoid almost certain problems that would arise due to EDS, avoid a trip to the ER, avoid being charged for the trip to the ER, avoid causing Emily more pain and suffering, etc.

So then I, huffily, I am sure, asked about the needle. She told me that they didn’t have the atraumatic type and the doctor didn’t think it would make a difference anyway. By this time I am livid…. Halfway tempted to tell them to stop and refer us to somewhere else that can meet Emily’s needs and I told the nurse so. And, that with all due respect, I disagreed with the doctor’s opinion and that I did indeed believe that the atraumatic needle is necessary. I was very unhappy and I wanted to walk away, but, in my heart, I knew she needed this procedure done and that it could wait another week or two to reschedule it.

Then the doctor comes in and introduces herself and tells me she will be using the special needle. I immediately, and understandably, assume that I am being lied to because I have just been told otherwise. But again, I feel trapped – not because it would be an inconvenience to stop the procedure, but by sheer need. And I did not believe I was going to get a straight answer even if I made an issue of being lied to. Then I had to leave my medically complex child on the table with people who I believed were lying to me. This may be the way things are done, but this did not help my mental state.

My hubby popped down (he works at the hospital) to check on us and he found a very unhappy mama fuming in the waiting room. He had to get back to work before she was done so I was alone when the doctor came out to talk to me.

Turns out she wasn’t lying after all – she did actually use the atraumatic needle. She said she didn’t like it, it felt weird going in, although it worked fine. I don’t know if the nurse had told her I was mad about it and she decided to use it or if she was planning on using it all along, but… whatever. The nurse later apologized for telling me wrong, implying there was a lack of communication somewhere along the line. I tend to think the doctor changed her plan in light of my anger but I suppose I will never know.

She had to lie flat in recovery for an hour, but, and this is the third EDS specific request I made, I told them she was going to need to be in recovery for longer than normal. No matter how many times I said this, they still started pushing her out the door. At about an hour, they had her sit up – and she found that her head actually felt normal for the first time in a long time. Her head felt better after the LP than it did before. Her back, not so much. In the end, I could not fight both the nurse and Emily who said emphatically, “I am starving. Get me out of here and feed me.”

So we left, I fed her and she went home to lay flat for the rest of the day.

Anyway, after all was said and done, we did fortunately get some valuable information from the LP. You may have already guessed that by her headache being relieved by the LP that she did indeed have some high pressure going on. Her opening pressure was 20.2. The doctor told Emily that normal should be between 10 and 15; I read the range is 7 – 18. Either way, Em had high pressure, although she is on Diamox three times a day. She had taken her morning dose 2 hours before the LP, which means it basically should have been reaching its peak effectiveness when the LP was done.

We have our proof as far as I am concerned. I would be fascinated to know what her pressure would have been without Diamox – not that I am willing to find out, mind you. But, my guess is it would be quite high. In fact, the symptoms that had been plaguing her: headache, unstable neck, blurred vision, light/ sound sensitivity, even that issue with her airway, all improved dramatically when the pressure was relieved. Exactly like it did September 19, 2012 when she took her first dose of Diamox.

Once we knew that there absolutely was high pressure causing the headache, the burning question became: is her pressure high because her dose needs increased or because her CO2 is low, thus making the Diamox ineffective? Hubby was able to call that afternoon and get her CO2 level – 26. It needs to be 21 or higher. This clearly is not the issue. We don’t have all of her bloodwork back yet, but that was the important one at the time.

Oddly, she had what we think might have been another CSF leak Saturday night, in her bad ear. She was laying on the side with the ear that has been problematic for months now and suddenly said that her ear was wet. I declined sticking my finger in her ear like she asked me to but her hair was damp where she had been laying. And she got a tissue and, after laying on it for a while, it was damp. But, it cleared up quickly, we think, and we saw no more signs of a leak.

However, this leak may have added to the problems that were to follow. If that sounds ominous to you, you are on the right track.

Part 2, in which we unsurprisingly and very predictably end up in the ER and have LOADS of fun, is coming up soon…

As we waited to get to the concussion experts – and fortunately we did not have to wait long – Emily’s concussion symptoms remained concerning. She is having trouble reading and comprehending, she struggled to write – to spell and remember the mechanics of language. As we passed the 3 week mark of her head injury, she was not getting better and, while she was perhaps not getting worse, more deficits were being revealed. I am not sure we even know the extent of her deficits at this point. When she is searching for a specific word, we go through a very entertaining version of verbal charades where she tries to make me understand what she is trying to say, with sometimes hard to follow logic and obscure literary references. Yesterday, she tried to use a story from the Little House on the Prairie books, to get to the word “fraternize”. We got there but, man, it was a circuitous journey! As always, we are still able to laugh at ourselves and the situation.

At the Concussion Clinic, we saw a rehab doctor, a neurologist and a doctor who I believe was a fellow, which is very common in our experience at Children’s. The exam revealed that her short term memory is crap, to put it plainly. She was given 3 words to remember. Maybe 5 minutes later she was able, with some effort, to pull up the first one, “green”. She was eventually able to guess the second word, “liberty” when given a prompt that it was another word for freedom. She could not recall the third, “automobile” even when given a prompt that it was a word for getting around. She guessed “car”, was told it was something like that and guessed “bus”. She remembered the word when he said it. She also took a fair amount of time to be able to say when her birthday is, which is not normal and is something she answers every single time she is at the doctor.

Her balance is off – she struggled to walk a straight line. Her eyes are not right – she either showed nystagmus or saccadic oscillations/ interruptions. (Her eyes were jerky when tracking side to side; I was able to clearly see them from where I was sitting. There was some discussion about this – I was familiar with nystagmus but had to look up “saccadic”. I was able to do so, even though it was a totally unfamiliar word, because we had a funny moment when Emily thought the doctor called her “psychotic”, “saccadic” does sound similar. He laughed and told her he would never call her psychotic to her face. We liked him quite a bit. 🙂 So, I was able to find what they said, I just don’t know the significance of it.)

So, they all agree that she did have a concussion and she has post concussive symptoms. Likely the fact that she (probably) had a CSF leak made her brain extra vulnerable to injury at the time because there was less cushion to protect it. They were concerned with the Diamox contributing to her CSF volume, but she was not taking it at the time of her injury. It was a day or two later that she started taking it again. They have referred us to speech therapy where they will assess her and do cognitive therapy to help get her brain back in shape. We are going to do that at our local hospital and they were fine with that. We will go back in a month and they will do neuropsych testing to further evaluate her, particularly if therapy is not helping as much or as quickly as we would like.

They were understandably concerned with the number of meds she is on. Frankly, they can join the club. Of course we are concerned and, yet, this is what has made her functional and removing any of them seems insane. They didn’t push that and said they would not mess with her meds since she has other docs overseeing them. I was thankful for that. Very thankful. Particularly that they left the Diamox alone.

The neurologist, through no fault of her own, should have come with a trigger warning. She was nice enough but she had the same basic advice that we got at the headache clinic at Cincy 4 years ago: eat your vegetables and drink your milk. Once again, her point is absolutely correct, as it was 4 years ago. Eating well and nourishing your body is vital to wellness. Unfortunately, that gets a little complex when one has a handful of medical conditions that make eating/digesting hard. Furthermore, when we mention that Emily is lactose intolerant and that detail is ignored because it goes against their standard protocol, it makes her a little tiny bit furious. That day, she was exhausted, hurting, scared and now this “chick” is telling her to drink milk. That said chick was a little heavy on barking orders and light on (visible) compassion did not help. Em has a, ahem, less than favorable view of her but, I know she was just doing her job. And we have the same goal – to help Em. Once they left Em let loose about her and didn’t even laugh at my favorite medical joke: How are God and a neurologist different? God doesn’t think he is a neurologist. She did say she wanted to go give her neurologist at home a hug for not being a jerk and taking good care of her. And, presumably not telling her to drink milk.

We were suitably impressed with them over all; I feel like she is in good hands. However, I am not sure they truly understand her complexity. My guess is they don’t see a ton of (diagnosed) EDS patients in their clinic and so would have little reason to be familiar with it. I mean, you can understand the general mechanics of connective tissue and not fully grasp the entirety of what EDS will do to a person or how the simplest thing is a huge challenge.

They knew her shoulder was bad, she was wearing a sling – the MRI report was in her records, but it was like they didn’t quite grasp how unstable it is, how much it hurts and how much the CRPS pain affects her. The Rehab Doc was concerned over the tightness/knots in her neck and shoulders, because that typically is seen with concussions and contributes to the headache. What we were not able to really communicate is that that is not new – that is her baseline. And when we relieve those knots or muscle spasms, the adjoining joints often are unstabilized. Could that tension be contributing to her “concussion headache”? Sure. Is it going to be easily managed? Probably not. He wanted her to do a few stretches, some of which she cannot do because of her shoulder. In the end, he told us to talk to her PT and have him work on addressing it. The PT was incredulous that the doc would have her doing anything with her hypermobile neck and said, “Look, I don’t want to tell you to he is wrong, but don’t do any of that. We have to protect your neck. And you can tell him I said so.”

Once again, we are trying to navigate this ocean of conflicting advice. We just do our own research, try to know what we need to know and make an educated decision. We tend to go with the advice that makes the most sense at any given time and aligns the most with our understanding of all of the pieces of the puzzle. We also tend to go with the advice from the professionals who know her best.

So, the situation is at least as serious as I feared and perhaps more. It was really hard to see her struggle and see the fear and frustration on her face when asked to do a task she couldn’t do. But, concussions can take months to recover from and it is entirely possible that we will see her brain heal spontaneously over the next few weeks. Therapy will help and my husband, as always, has handpicked the best therapist for Em and her situation. We are optimistic and hopeful that she will get better in the near future.

Continued in Part Three: Shoulder…. Because that is a thing. Just not quite as big a thing as her brain.

Right now the medical issues are coming at us faster than we can field them. What was of the utmost importance two months ago is now on the back burner as bigger problems arise. I think it is safe to say we are in survival mode. I don’t quite know where to start as I sit down to post. I haven’t had time to even consider posting with all of Em’s medical issues and trying to survive the holidays and now there is a lot of catching up to do.

So, this post will be Part One: Brain Related. Part Two will be the rest of that story and Part 3 will be shoulder related and will be posted tomorrow. Otherwise, it would be an amazingly long post, even by my standards and that is a pretty high standard!

Last I posted, Em had been to the ER for a suspected CSF leak. Since then, she saw the ENT for her ongoing ear infection. She did have a fungal infection, which he treated. He was concerned about the idea of a CSF leak and seemed to immediately grasp the risk for her, which was a comfort. We see him again on the 19th to follow up. Her ear is hurting again so we may not make it that long.

She also saw the neurologist to follow up after the ER visit. We couldn’t get in for almost 2 weeks and in the meantime, the low pressure symptoms abated and we assume, if there was a leak, and I think there probably was, it healed. He wanted to do a spinal tap to rule out infection and check her pressure but we talked him out of it. Her symptoms were better and it did not seem wise to subject her to it, not with everything else going on, which will become clear in a moment. He understood and put a note in her chart to schedule her for a spinal tap if we call with further symptoms. That way we don’t have to go through the ER and hope they do something about it. LPs are not to be undertaken lightly for people with connective tissue disorders and, while I understand we may end up at a point of needing one, we want to avoid them if at all possible.

The biggest concern by the time we got to him, was not so much the low pressure issue because that seemed to level out, but very concerning symptoms after a head injury she experienced the day after going to the ER. It was a relatively simple, mild injury: the shower head fell on her head while she was showering. For anyone else, it would have been no big deal. For her, it has turned into a scary episode. It is perhaps complicated by the fact that she has hit her head several times in the last couple months. It was also complicated by the fact that she likely had a CSF leak and so her brain had less cushion to protect it.

Initially, the head injury was mixed up with all of her other stuff (bad reactions to new meds, low pressure/ CSF leak symptoms, even cognitive issues from CRPS) and in a way it didn’t matter because the treatment would just be to rest and allow her brain to heal, which she did. It seemed like such a minor injury that the ER would flag us as nuts if we took her in, particularly since she had been there just the day before. (“Hi, we are back. I know she just had a CT scan yesterday but she was attacked by the shower head so can we have an MRI?” Yeah, that would get us flagged for sure.) But as we got a day or two away from the injury, I started getting more concerned and had to wonder if she really did have a concussion. Like, maybe a serious one.

Then, 5 days after her injury, we had a conversation that made me realize it was more serious than I already thought. She told me we could have a conversation but she wouldn’t remember it. So, I probed and tested her memory. She could not remember how old she was going to be on her birthday on the 22nd. “Am I going to be 17 or 18?” She couldn’t remember when we were going to celebrate her birthday. I still am unclear if she didn’t know when her birthday was or just didn’t know when everyone was going to be present to celebrate. She couldn’t remember details about her upcoming Ortho appointment that I had told her an hour before. She couldn’t remember what day her brother was flying home for Christmas. Both were important events; notable to her. She couldn’t remember things that had happened the day before at her ENT visist. Over the next few days, as we waited to get to the neurologist, her memory remained spotty and concerning. My observation was that she could sometimes remember when prompted and reminded or that she guessed instead of knowing the answer, often wrong.

So, when we talked about it with the neurologist, he said it sounded like a concussion and he wanted us to see a concussion expert. I was able to get her an appointment at the Concussion Clinic at Cincinnati Children’s on the 5 and it is a good thing we made it a priority.

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So… another installment of Em and Beth’s excellent adventures. (Doesn’t quite have the same ring as “Bill and Ted’s” but whatever…)

New Meds

Since I last posted, Em has had to go off the Cymbalta. She has been on several meds that, combined, can cause Serotonin Syndrome, and Cymbalta was one more to add to that list. When she displayed confusion, lethargy, and worsening muscle twitching, we got concerned. The doctor wasn’t – but I was and so I called the pharmacy. The guy I talked to spent a fair amount of time on the phone with me and finally said if it was his kid, she would not take the new med anymore. Which sealed the deal for me. Nucynta is another one that can be a problem, but in a much lesser way than the Cymbalta, so we are hopeful that she can perhaps try it once the Cymbalta is out of her system and maybe it will help. Again, it is a thing that it was helping her pain (although she didn’t actually notice much relief when she had taken it, but rather when it wore off) but she just can’t tolerate it. That gets heartbreaking, to be honest.

I actually haven’t talked to the pain doc about stopping the med – we made an “executive decision” to stop it. He wasn’t a bit concerned when I talked to him about the worrisome symptoms, which is rather concerning to me, if I am honest. I know he is just trying his best to help and sometimes we have to take calculated risks. This just is not one that worked out.

Because he sort of blew off my concerns, I am hesitant to call back and tell him that we decided it best to take her off the med. I don’t think it will be a problem but I just haven’t had the time or inclination to jump back into that fight, for reasons that will be apparent in a moment.

Another Trip to the ER

We are thinking that Em has developed a CSF leak and made a trip to the ER yesterday at the advice of the neurologist. Or, more accurately, the nurses at the neurology office because they didn’t get around to returning my second call until the end of the day when they couldn’t get a hold of the doctors.

It was Dr. Diana that suggested the possibility of a leak – that perhaps the weirdness with her ear was due to a leak. (Thank God for Dr. D, I don’t know where we would be without her.) Now, all of these new and worrisome symptoms were sort of mashed up together, but after she stopped the Cymbalta on Tuesday, she started complaining of a headache that went away when she laid down and quickly got horrible when she is upright. So, she had already been in bed for a few days because the Cymbalta knocked her out. But, starting Monday or Tuesday, every time she sat up, the headache was awful and when she lays down it goes away. So, that seems like a low pressure headache and/ or a leak. Who knows how long it would have taken me to figure it out without a helpful word from Dr. D?

Diamox made it worse, so we pulled back on it and let her miss a dose to see what happened. Her head got a little better, but still, she couldn’t be upright. And, there was the concern of meningitis. So, I called the office late on Wednesday, called back on Thursday when I didn’t hear back and they finally got back to me and they said to go on to the ER.

You can imagine that this was not well received after our last trip to the ER. Understandably, we were both anxious about how it would go.

Fortunately, this trip was much better. They took her pain and symptoms seriously and treated her well. They started an IV, ran some fluids and gave her some pain medication. It took the edge off of her headache and let her rest a little. They did a CT scan, which found no big leaks, but could not rule out a small leak. They could not do the test to check for a leak at that time of night. I am not sure what he was referring to there, but we will follow up with the neurologist and pursue it further.

He said there was no indication that she has meningitis but the only way to tell for sure would be to do a spinal tap. He did not recommend it and we all agreed. Wholeheartedly. And, he clearly didn’t feel a lumbar puncture was necessary which I was also in wholehearted agreement with.

He felt comfortable sending her home to sleep in her own bed and just follow up with the doctor, saying we can come back if it gets worse. I had packed to stay a few days if necessary (the last time we had a real emergency I didn’t and we ended up staying several days at Riley Children’s after a swift ambulance ride in the middle of the night and an appendectomy) and I was pretty darn glad it was unnecessary! If packing to stay is all it takes to get sent on home, I will do it every time from now on out!

This morning, her head is no better when she is upright. So, I think we are dealing with a low pressure headache. She took a small dose of Diamox yesterday afternoon before we went to the ER, but that is all she has had since Wednesday. She can make the call as to whether she needs it or not. And, we will play it by ear until we can get in to see neurology. I am also hoping that when we see the ENT, he might be able to help us figure out if her ear has anything at all to do with her headache. If it is a leak, with any luck it will heal itself and need no further treatment.

It is more than a little frustrating to have to work so hard to get answers – we spent hours in the ER and came away with no answers. And, yet, I am thankful that it was a positive experience, that they took her pain seriously and treated it, that they were caring and compassionate, and that we were able to rule the big stuff out. Of course, that leaves all the little stuff up for grabs, but that is our life.

When the doctor first examined her, he said he was going to go read her records and see if they could give him more insight into what was going on. When he came back in, a couple hours later, after the pain meds and CT scan, he sat down and said that he had been reading about Em and that it was enough to keep him busy for awhile.

How right he is! Having been kept busy for the last 6 years, I can tell you that truer words were never spoken.

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Welcome!

Thanks for visiting my blog where I chronicle 'Our Life with Ehlers Danlos Syndrome'. I am sharing my family's journey with EDS (and everything that goes along with it) in the hope that our experiences will help someone else.

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